Guillain-Barré Syndrome



Guillain-Barré syndrome is an “acute paralytic polyneuropathy” that affects the peripheral nervous system. It causes acute, symmetrical, ascending weakness and can also cause sensory symptoms. It is usually triggered by an infection and is particularly associated with to campylobacter jejuni,  cytomegalovirus and Epstein-Barr virus.

 

Pathophysiology

Guillain-Barré is thought to occur due to a process called molecular mimicry. The B cells of the immune system create antibodies against the antigens on the pathogen that causes the preceding infection. These antibodies also match proteins on the nerve cells. They may target proteins on the myelin sheath of the motor nerve cell or the nerve axon.

Presentation

  • Symmetrical ascending weakness (starting at the feet and moving up the body)
  • Reduced reflexes
  • There may be peripheral loss of sensation or neuropathic pain
  • It may progress to the cranial nerves and cause facial nerve weakness

Clinical Course

Symptoms usually start within 4 weeks of the preceding infection. The symptoms typically start in the feet and progresses upward. Symptoms peak within 2-4 weeks, then there is a recovery period that can last months to years.

Diagnosis

A diagnosis of Guillain-Barré syndrome is made clinically. The Brighton criteria can be used for diagnosis.

Diagnosis can be supported by investigations:

  • Nerve conduction studies (reduced signal through the nerves)
  • Lumbar puncture for CSF (raised protein with a normal cell count and glucose)

 

Management

  • IV immunoglobulins
  • Plasma exchange (alternative to IV IG)
  • Supportive care
  • VTE prophylaxis (pulmonary embolism is a leading cause of death)

In severe cases with respiratory failure patients may need intubation, ventilation and admission to the intensive care unit.

Prognosis

  • 80% will fully recover
  • 15% will be left with some neurological disability
  • 5% will die

 

Last updated April 2019
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